Name

Biologically active peptide(s) secreted

Incidence

Tumor location

Malignant %

Associated with MEN-1, %

Main symptoms/signs

ACTHoma

ACTH

Rare

Pancreas

>95

Rare

Cushing’s syndrome (100 %)

CRHoma,

Corticotropin-releasing hormone

Rare

Pancreas, lung

Unknown

Unknown

Cushing’s syndrome, rare

Serotoninoma

Serotonin, tachykinins

Rare

Pancreas

60–88

Rare

Diarrhea, flushing

Calcitoninoma

Calcitonin

Rare

Pancreas

>80

16

Diarrhea (50 %)

GHRHoma

Growth hormone-releasing hormone

Unknown

Pancreas, lung, jejunum, others

>60

16

Acromegaly (100 %)

GRFoma

Growth hormone-releasing factor

Rare

Lung, pancreas, others

30

<1 %

Acromegaly

PET causing hypercalcemia (PTHrp-oma)

PTHrp; others unknown

Rare

Pancreas

>90

Rare

Symptoms due to hypercalcemia

Ghrelinoma

Ghrelin

Rare

Pancreas, stomach, intestine

Unknown

Unknown

Starvation, anorexia nervosa, bulimia, cachexia

PET secreting renin

Renin

Rare

Pancreas

Unknown

No

Hypertension

PET secreting luteinizing hormone

Luteinizing hormone

Rare

Pancreas

Unknown

No

No anovulation, virilization (female); reduced libido (male)

PET secreting erythropoietin

Erythropoietin

Rare

Pancreas

100

No

Polycythemia

PET secreting IF-II

Insulin-like growth factor II

Rare

Pancreas

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